Patient: Female, 64Final Diagnosis: Segmental absence of intestinal musculatureSymptoms: Abdominal discomfortMedication: —Clinical Procedure: ColectomySpecialty: Diagnostics, LaboratoryObjective:Rare diseaseBackground:Segmental absence of intestinal musculature is a well described entity in premature infants. It presents with peritonitis, bowel perforation, and obstruction. The diagnosis is based on pathologic observation of absence of intestinal musculature. Researchers hypothesized that this entity is a result of a vascular accident during embryogenesis. However, segmental absence of intestinal musculature is no longer limited to the pediatric population. Recently, a few cases have been described in adults with and without significant vascular diseases. This change in the age of the affected population with segmental absence of intestinal musculature makes the understanding of the pathogenesis of this entity even more challenging.Case Report:Here, we report a case of segmental absence of intestinal musculature in a 64-year-old female. The patient presented to the emergency room with sudden onset of abdominal pain and signs of peritonitis. Abdominal computed tomography showed free air in the abdomen. Laparotomy was performed, and a perforation involving the descending colon was identified. Left hemicolectomy was performed. Pathologic examination of the resected colon showed segmental absence of intestinal musculature.Conclusions:Although the pathologic diagnosis of segmental absence of intestinal musculature is straightforward, the assumption that this condition is limited to the pediatric population is a major player in overlooking this diagnosis in adults. Pathologists should be aware that this condition can present in adults and is segmental. Gross and microscopic examination of perforated intestine is required to reach the correct diagnosis. To our knowledge, twelve cases of this entity have been described in adults. Here we present the thirteenth case of segmental absence of intestinal musculature in an adult, and we discuss the clinical and pathologic findings of this entity as well as its pathogenesis.

Patient: Male, 68Final Diagnosis: Urinary bladder angiosarcomaSymptoms: —Medication: —Clinical Procedure: TURBTSpecialty: Diagnostics, LaboratoryObjective:Rare co-existance of disease or pathologyBackground:Angiosarcoma is a fatal and aggressive mesenchymal tumor. It occurs in skin, breast, and parenchymal organs. It rarely arises primarily in the urinary bladder. Only 13 cases of primary urinary bladder angiosarcoma have been reported in the English literature.Case Report:The patient was a 68-year-old man who presented to the Emergency Department with inability to void. Computed tomography of the abdomen and pelvis showed a urinary bladder mass. Surgical excision of the mass was performed. Pathological examination results were consistent with angiosarcoma. In addition to the unusual location of this tumor, the pathology was different from the previously reported cases in that this case was rich with osteoclast-like multinucleated giant cells.Conclusions:The pathological diagnosis of primary urinary bladder angiosarcoma is challenging. Histological patterns and immunophenotypes are variable. Here, we review all reported cases of primary urinary bladder angiosarcoma, highlight the clinical and morphological features of this malignant neoplasm, and report a unique case of primary urinary bladder angiosarcoma with osteoclast-like multinucleated giant cells.

Angiomyolipoma is a mesenchymal neoplasm that originates from perivascular epithelioid cells and is characterized by benign proliferation of blood vessels, smooth muscle cells, and adipose cells. It occurs more commonly in women in the kidneys, and it is associated with tuberous sclerosis in about 30% of cases. Its clinical presentation ranges from being asymptomatic, to causing flank pain and hematuria, to being a cause of massive retroperitoneal hematoma. Rarely angiomyolipoma can involve extrarenal sites such as the liver, skin, oral cavity, and spleen. Here we report a case of intrabronchial angiomyolipoma obstructing the lower part of the main stem bronchus and producing left lung lower lobe collapse in a 69-year-old morbidly obese man not associated with tuberous sclerosis. Despite the presence of all of the 3 classic components of this entity, the diagnosis was challenging due to the unusual location and the absence of history of tuberous sclerosis. However the diagnosis was confirmed based on the histological features and the immunostaining pattern. To our knowledge, this is the third reported case of intrabronchial angiomyolipoma without tuberous sclerosis.